nephrosis 1

Question 1

what is the most common cause of nephrotic syndrome in children? What changes do you see?

Answer 1

MCD. No change by light microscopy. electron micro. shows podocyte changes (effacement and fusion and sometimes villous transformation)

Question 2

What causes the changes seen in MCD?

Answer 2

disruption of SGPs leads to loss of neg. charge and then fusion of fp and aggregation of actin filaments

Question 3

Diabetic nephropathy: pathogenesis

Answer 3

non enzymatic glycation of proteins, decreased proteoglycans, increased TGF-beta and ROS. Thickened GBM and TBM traps proteins and GFR declines

Question 4

describe affect of DM on GFR

Answer 4

at first it increases (superhyperfiltration) due to poor HS-PG depostion->^pore size and decrease charge(->proteinuria). Then it declines due to protein trapping.

Question 5

describe the proteinuria in DM

Answer 5

Initially (microalbuminuria) the selectivity for albumin is high then as you get more proteinuria its less selective

Question 6

what meds do we prescribe in pts with early signs of diabetic nephropathy? Why?

Answer 6

ACEI. it reduces superhyperfiltration which damages glomerulus

Question 7

Describe amyloid

Answer 7

inert fibrilar protein. criss-cross pattern. 8-10 nm thick. Birefringent due to beta-pleated sheets

Question 8

what happens in amyloidosis?

Answer 8

proteins precipitate and deposite in mesangium w/o hypercellularity

Question 9

Focal segmental glomerulo-sclerosis: what type of proteinuria? History of? what glomeruli? What happens? what gets deposited? Special cells/

Answer 9

nonselective. H/O MCD, Heroin, HIV, Ureter reflux. Juxtamedullary. SEGMENTAL deposits of IgM and C1q in hyaline segments. Foam cells (lipid in mesangial cells)

Question 10

How does a low protein diet help in diabetic nephropathy?

Answer 10

you decrease the hyperfiltration and the proteinuria

Question 11

Idiopathic membranous nephropathy (IMN): H/O? What deposits and where?

Answer 11

neoplasms, hepatitis, tx w/ gold or penicillamine. IgG and C3 immune-complex deposits in SUBEPITHELIUM, NOT mesangial matrix (as in lupus)

Question 12

what are two subsets of IMN?

Answer 12

Antigen-excess dz, Heymann nephritis (autoab’s against megalin and antiphospholipase A2 receptor)

Question 13

SLE: first signs? other signs?

Answer 13

butterfly rash; proteinuria, hematuria, casts

Question 14

Two principle presentations of SLE?

Answer 14

Diffuse (nephritic) and membranous (nephrotic). Diffuse has worse prognosis due to lots of abnormal serology (more ANAs, circulating immune complexes, cryo Igs, etc)

Question 15

hwo can lupus nephritis progress?

Answer 15

nephrotic->nephritic but not other way around.

Question 16

membranous lupus GN: characteristic glomerular changes. Where do complexes deposit

Answer 16

thick GBM w/ immune deposits. Karyorrhexis (fragmenting nuclei in areas of hypercellularity). subepithelial and mesangial deposits.